Single dominant lesion in capillary malformation-arteriovenous malformation (CM-AVM) RASA1 syndrome.

We report two cases with localized vascular malformations clinically resembling the "dominant lesion" seen in capillary malformation-arteriovenous malformation (CM-AVM) syndrome, however, lacking germline RASA1 variants but presenting double somatic RASA1 variants in affected tissue. Both patients presented with localized and superficial high-flow vascular malformations were treated with surgery and laser therapy and showed partial resolution. The study underscores the rarity of somatic RASA1 variants, contributes to understanding the "second-hit" pathophysiology in vascular lesions, and emphasizes the significance of clinical distinctions and genotyping for accurate diagnoses, offering implications for diagnosis, prognosis, and genetic counseling.

Three-dimensional planning in craniomaxillofacial surgery.

INTRODUCTION: Three-dimensional (3D) planning in oral and maxillofacial surgery has become a standard in the planification of a variety of conditions such as dental implants and orthognathic surgery. By using custom-made cutting and positioning guides, the virtual surgery is exported to the operating room, increasing precision and improving results. MATERIALS AND METHODS: We present our experience in the treatment of craniofacial deformities with 3D planning. Software to plan the different procedures has been selected for each case, depending on the procedure (Nobel Clinician, Kodak 3DS, Simplant O&O, Dolphin 3D, Timeus, Mimics and 3-Matic). The treatment protocol is exposed step by step from virtual planning, design, and printing of the cutting and positioning guides to patients' outcomes. CONCLUSIONS: 3D planning reduces the surgical time and allows predicting possible difficulties and complications. On the other hand, it increases preoperative planning time and needs a learning curve. The only drawback is the cost of the procedure. At present, the additional preoperative work can be justified because of surgical time reduction and more predictable results. In the future, the cost and time investment will be reduced. 3D planning is here to stay. It is already a fact in craniofacial surgery and the investment is completely justified by the risk reduction and precise results.